Immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) is a relatively new disease entity, thus very limited data are currently available regarding its epidemiology. To date, several case series regarding clinical profiles of IgG4-SC have been published, including our study [1–3]; however, no epidemiological studies illustrating the incidence and prevalence of IgG4-SC have been conducted. In this review, epidemiological and clinical features of IgG4-SC are discussed in general and are based mainly on data in the Japanese population.
- 2.Huggett MT, Culver EL, Kumar M, Hurst JM, Rodriguez-Justo M, Chapman MH, et al. Type 1 autoimmune pancreatitis and IgG4-related sclerosing cholangitis is associated with extrapancreatic organ failure, malignancy, and mortality in a prospective UK cohort. Am J Gastroenterol. 2014;109:1675–83.CrossRefPubMedPubMedCentralGoogle Scholar